Late vitamin K deficiency bleeding in an infant with choledochal cyst.
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Infantile choledochal cyst (CC) usually presents as jaundice, vomiting, acholic stools, and hepatomegaly, and it can resemble biliary atresia. Although bleeding tendency is a rare clinical presentation of CC, it can be the first symptom, especially in infants less than 12 months of age. We report a case of a two-month-old infant with choledochal cyst presenting as late vitamin K deficiency bleeding (VKDB). Early recognition of diseases predisposing to VKDB and immediate investigation and treatment of warning bleeds help to prevent the worst consequences. Late VKDB is often the presenting feature of a serious underlying disease that may be recognized early. The sudden onset of bleeding tendency in infants with congenital liver or biliary tract disease may suggest not only biliary atresia but also, although extremely rare, CC. Early vitamin K administration leads to rapid normalization of hemostatic parameters, which enables major liver surgery. (+info)
Choledochal cysts are rare cystic dilatations of the bile ducts, which are commonly found in children. Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct (type VI choledochal cyst) is a unique occurrence. To date, specific pathogenic and diagnostic criteria for describing type VI cysts have been lacking. This report attempts to lay down the specific diagnostic and management criteria of this uncommon entity, with reviews of all available cases reported in the literature. (+info)
Complications of choledochal cysts in adulthood.
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Choledochal cyst is a well-recognised entity, presenting primarily in infants and young children. Where symptoms are delayed until adulthood, associated hepatobiliary pathology may complicate the presentation. These problems may be aggravated by previous treatment with bypass surgery rather than resection. We report seven cases from our recent experience presenting with complications in adulthood. These included cholangitis, hepatic abscess, pancreatitis and malignancy within the cyst. Two patients presented during pregnancy. These complications and their implications for management are discussed. (+info)
Hydatid cyst of the pancreas mimicking choledochal cyst.
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Management of choledochal cyst with portal hypertension.
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Portal hypertension (PHT) is a rare complication associated with choledochal cysts. Management issues of PHT patients are inadequately addressed, as its incidence is low and underlying causes variable. We report three cases of choledochal cyst with PHT. All patients had type IVa choledochal cysts, and the causes of PHT were secondary biliary cirrhosis (SBC) (two cases) and alcoholic liver disease (one case). Clinical presentation included jaundice, gastrointestinal bleeding and ascites. One patient with SBC successfully underwent excision with Rouxen-Y hepaticojejunostomy, while the patient with cholangitis was managed with endoscopic retrograde cholangiopancreatography stenting. The last patient with alcoholic liver disease was managed conservatively for seven years and died of liver failure. Management of choledochal cysts depends on the severity of liver disease in cases of cirrhosis of unrelated cause, while those with SBC should be considered for surgical management. Endoscopic stenting may be considered as a temporary measure in high-risk cases. (+info)